Macular retinoblastoma: evaluation of tumor control, local complications, and visual outcomes for eyes treated with chemotherapy and repetitive foveal laser ablation.

Objective: To determine tumor control rates, complication rates, and visual acuity (VA) for patients with macular retinoblastoma undergoing systemic chemotherapy and foveal diode laser ablation.

Design: Noncomparative retrospective case series.

Participants: All patients with retinoblastoma in the macula at the Bascom Palmer Eye Institute between 1991 and 2004 were evaluated.

PRCC-TFE3 renal cell carcinoma in a boy with a history of contralateral mesoblastic nephroma.

The genetics of renal tumors in children is widely recognized. However, most of the studies published to date emphasize the association between Wilms tumor and the WT-1 gene. Recently, a unique translocation between the X chromosome and chromosome 1 or t(X;1) has been described in several reports of renal cell carcinomas (RCCs) diagnosed in children and adolescents that results in PRCC-TFE3 gene fusion.

Ganglioneuroblastoma metastatic to the orbit.

A 10-month-old girl presented with an extensive orbital and cranial metastatic lesion from an adrenal ganglioneuroblastoma. Treatment with chemotherapy alone resulted in complete regression of the tumors with over 7 years of follow-up. Good prognostic indicators included her young age at diagnosis, DNA index of tumor cells of 1.

Eyelid and scleral necrosis following bare iridium-192 seed for retinoblastoma.

A 2 1/2-year-old girl with a history of bilateral retinoblastoma underwent primary enucleation of the right eye and was referred for further management of persistent tumor in the fellow eye. Previous treatment of the left eye included external beam radiotherapy, systemic chemotherapy, laser photocoagulation, cryotherapy, and direct scleral application of a bare iridium-192 radioactive seed. Examination revealed focal full-thickness necrosis of the left upper and lower eyelid and a large inferonasal viable retinoblastoma tumor with overlying retinal detachment.

Comparison of retinoblastoma reduction for chemotherapy vs external beam radiotherapy.

Objective: To determine the time course and extent of tumor reduction associated with systemic chemotherapy or external beam radiotherapy (EBRT) in the treatment of advanced intraocular retinoblastoma.

Methods: Retrospective review of children with Reese-Ellsworth stages IV and V retinoblastoma undergoing primary globe-conserving therapy with either systemic chemoreduction or EBRT. Study variables were recorded at baseline, at monthly intervals for the first 6 months, and at 12 months after the initiation of treatment.

Treatment of recurrent eosinophilic granuloma with systemic therapy.

Purpose: To describe the systemic treatment of recurrent eosinophilic granuloma.

Methods: Case report and review of Langerhans cell histiocytosis.

Results: A 9-year-old boy had painful, right upper eyelid swelling.

Hematogenous brain metastasis in children.

Hematogenous brain metastases are uncommon in childhood. Three patients and a literature review that includes centers reporting up to 36 years of experience are presented in this study. The total of 2,040 patients includes our three examples of one neuroblastoma, one hepatoblastoma, and one adrenal carcinoma.